Interstitial Pneumonia with Autoimmune Features (IPAF)

ZUSAMMENFASSUNG

Der Terminus Interstitial Pneumonia with Autoimmune Features (IPAF) wurde im Jahr 2015 samt entsprechender Klassifikationskriterien von einer interdisziplinären Task Force bestehend aus Mitgliedern der European Respiratory Society (ERS) und American Thoracic Society (ATS) vorgeschlagen. Das Akronym bezeichnet keine eigenständige Entität, sondern umschreibt ein heterogenes Patientenkollektiv mit idiopathischen interstitiellen Pneumonien und autoimmunen Eigenschaften. Für die Klassifikation einer interstitiellen Pneumonie als IPAF gilt: 3 Vorbedingungen und 2 von 3 Domänen (1. Klinische Domäne, 2. Serologische Domäne, 3. Morphologische Domäne) müssen erfüllt sein. Eine Domäne gilt als erfüllt, wenn mindestens ein Kriterium nachgewiesen wird (siehe Kasten „Klassifikationskriterien“). Die IPAF-Kriterien wurden geschaffen, um Patienten dieses Kollektivs für die weitere Erforschung in standardisierter Weise zu klassifizieren. Bislang gibt es jedoch sehr wenige prospektive IPAF-Beobachtungsstudien und die verschiedenen IPAF-Kohorten sind äußerst inhomogen. Sowohl die Klassifikation als auch die Betreuung von IPAF-Patienten erfordern eine interdisziplinäre klinische Zusammenarbeit zwischen Pneumologen und Rheumatologen.

ABSTRACT

In 2015, the term “Interstitial Pneumonia with Autoimmune Features” (IPAF) and classification criteria were proposed by an interdisciplinary task force with members of the European Respiratory Society (ERS) and the American Thoracic Society (ATS). The acronym does not denote an independent entity but describes a heterogeneous patient collective with idiopathic interstitial pneumonia and autoimmune features. For the classification of interstitial pneumonia as IPAF, 3 preconditions must be met. In addition, 2 of the following 3 domains must be fulfilled: 1. Clinical domain, 2. Serological domain, 3. Morphological domain. To fulfil a domain, proof of at least one criterion is sufficient (see box). The IPAF classification criteria were created to classify patients of this collective for further research in a standardized manner. So far, however, there are unfortunately very few prospective IPAF observational studies and the different IPAF cohorts are extremely inhomogeneous. Both the classification and care of IPAF patients requires interdisciplinary clinical collaboration between pulmonologists and rheumatologists.

Schlüsselwörter

IPAF - interstitielle Pneumonie Autoimmunmerkmale

Keywords

IPAF - interstitial pneumonia autoimmune features

Publication History

Article published online:
05 November 2022

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